What Is Idiopathic Pulmonary Fibrosis? IPF Part 1

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PATIENT EDUCATION | INFORMATION SERIES PATIENT EDUCATION | INFORMATION SERIES What Is Idiopathic Pulmonary Fibrosis? IPF Part 1

Pulmonary fibrosis literally means scarring Bronchioles (small airways) of the lungs. The scarring occurs in the interstitium, which is the lung tissue that Alveoli (air sacs) provides the scaffolding (support structure) for the alveoli (air sacs). Fibrosis thickens Alveoli with blood vessels Scarring in alveoli prevents the interstitium, making the air sacs stiff surrounding each air sac oxygen from passing into so that they are unable to fully expand and blood vessel. CO2 CO2 hold as much air as they normally should. O2 O2 This thickening also limits passage of oxygen through the wall of the alveoli into the bloodstream. Over time, fibrosis can worsen to Scarring (ﬁbrosis) the point that patients may need supplemental oxygen to raise low blood oxygen levels, relieve shortness of breath, and improve Inside of exercise ability. This fact sheet will describe IPF O2 alveoli O2 and how it is diagnosed—for more information Interstitium Interstitium on the Treatment of IPF see Parts 2 and 3 at Scarring (ﬁbrosis)

CLIP AND COPY AND CLIP www.thoracic.org/patients. Inside of CO blood CO 2 vessel 2

Pulmonary fibrosis can happen for many different Is IPF a rare disease? reasons, including autoimmune disorders, environmental IPF is a rare disease, but it is still common enough that you or occupational exposures, as a side effect of certain may know someone with this diagnosis. Overall, about 1 medications, and a variety of other causes. In many out of every 5,000 people has IPF, which equates to about cases, despite extensive evaluation, the cause is 50,000-100,000 people in the United States, though data unknown; we call such cases idiopathic. It is important from 2015 shows that as many as 200,000 people may be that you have a full evaluation by an experienced lung living with the disease. IPF is much more common in older specialist to rule out other causes of pulmonary fibrosis age groups. In people over 65 years of age, up to 1 out of before being diagnosed with idiopathic pulmonary every 200 people have IPF. fibrosis (or IPF). While the cause of IPF is unknown, it is a What are the signs and symptoms of IPF? form of pulmonary fibrosis, and specific criteria must be The main symptom of IPF is shortness of breath with met before the diagnosis of IPF is made. activity, which exists in almost everyone as the disease How does IPF develop and who is at risk? progresses. In fact, in IPF, shortness of breath often limits IPF is usually diagnosed in people between the ages of 50 physical activity. People with IPF may have to slow down, and 80 years. IPF is very uncommon in people under the age rest and recover throughout an activity or even give up of 50 years. Men are at higher risk than women. A past or physical activities they once did with ease. Many people current history of smoking cigarettes or working in a dusty with IPF notice they have the most trouble walking environment also increases the risk of developing IPF. IPF quickly, climbing stairs, or going up inclines. About 85% sometimes runs in families, and there are several genes of people with IPF will have a cough. The most frequently- associated with its development. occurring symptoms of IPF (shortness of breath, cough and

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PATIENT EDUCATION | INFORMATION SERIES fatigue) are also typical symptoms of other more common Is IPF a serious disease? diseases; thus, other more common conditions may need IPF is a serious disease. Most people with IPF will have to be ruled out before a diagnosis of IPF is made. shortness of breath, exercise limitation, and cough as By definition, IPF affects only the lungs. If a person has joint the disease progresses. Many people will require oxygen stiffness or inflammation, rash, or symptoms coming from at some point in their life. On average, IPF shortens a outside the lungs, then another condition might be present person’s life. In previous studies, half of people with IPF that requires special testing. In late stages of the disease, live less than 3-5 years, and half live longer than 3-5 years. IPF can put a strain on the right side of the heart. This is Some people may have lung transplantation for advanced called pulmonary hypertension, which can be associated disease. Over the last decade, advances in the management with swelling in the ankles and worse lung symptoms. of IPF have improved the outlook for patients. There are FDA medications approved for IPF and many clinical trials How is IPF diagnosed? attempting to find new therapies. The diagnostic evaluation begins with a thin slice chest CT scan (computerized x-ray imaging). A pulmonologist (lung Authors: Sabina A. Guler, MD, MHSc; Kathleen O. Lindell, PhD, specialist) with specific expertise in pulmonary fibrosis will RN; Jeff Swigris, DO, MS; Christopher J. Ryerson, MD, MSc review your symptoms, medical history, past medication Reviewers: Bridget Collins, MD; Amy Hajari Case, MD, use, and occupational history. Potential exposures that FCCP; Naftali Kaminski MD; Marianna Sockrider MD, DrPH; cause lung scarring in the home or workplace will be Charlie Strange, MD, ATSF discussed. A history of pulmonary fibrosis in other family members is an important clue to the diagnosis. Often your doctor will hear crackles in your lungs when listening with a R Action Steps stethoscope. Your doctor should also send you for blood tests that can ✔ If you have shortness of breath or cough that be used to identify blood markers of autoimmune diseases. does not go away, talk with your doctor. Currently, there is no single blood test or genetic test to ✔ Do not smoke or vape and ask your doctor for diagnose IPF. Pulmonary function tests (breathing tests) support if you need help stopping. and tests of oxygen levels (e.g., walking tests) are often ✔ If you have been told you may have IPF, ask to used to rate the severity of IPF and to look for worsening see a lung specialist who has experience with its over time, but these are not helpful in actually diagnosing IPF, as they can be abnormal in other conditions as well. An diagnosis and treatment. echocardiogram (ultrasound of the heart) can be used in Healthcare Provider’s Contact Number: more advanced IPF to evaluate whether there are any heart complications, such as pulmonary hypertension. Some people will need additional tests to make a diagnosis, including flexible bronchoscopy or a surgical lung biopsy. Bronchoscopy is a procedure in which a thin flexible tube For More Information is passed into the lungs through the mouth or nose usually American Thoracic Society done when a person is sedated (asleep). Samples can be • www.thoracic.org/patients/ taken through this tube that can be sent for various tests. – Flexible bronchoscopy (For more information, see ATS Patient Information Series – Oxygen therapy on Flexible bronchoscopy at www.thoracic.org/patients.) A – Part 2: Medications for Idiopathic Pulmonary surgical lung biopsy is a more invasive procedure in which Fibrosis two or three small incisions are made in the side of the – Part 3: Nondrug Treatments for Idiopathic chest under general anesthesia. During this procedure, a Pulmonary Fibrosis surgeon takes two or three samples of lung tissue that can Pulmonary Fibrosis Foundation be looked at under a microscope. • https://www.pulmonaryfibrosis.org/ Making a final diagnosis of IPF typically requires a lung specialist who has experience in evaluating and treating National Heart Lung and Blood Institute (NHLBI) patients with pulmonary fibrosis. Patients are also usually • https://www.nhlbi.nih.gov/health-topics/idiopathic- discussed in a multidisciplinary conference, in which various pulmonary-fibrosis clinical features, imaging findings, and biopsy results are reviewed in a group discussion. This discussion most often This information is a public service of the American Thoracic Society. The content is for educational purposes only. It should not be used as a includes a pulmonologist, radiologist, and pathologist. substitute for the medical advice of one’s healthcare provider.